Adolescent and Young Adult Cancer Survivors in Primary Care
- Jennifer L. Reichek, MD
- Attending Physician, Hematology/Oncology, Ann & Robert H. Lurie Children's Hospital of Chicago; Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine
- Disclosure: Dr. Reichek has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion. Read Dr. Reichek's profile
- Karina Danner-Koptik, RN, MSN, APN, CPON
- Clinical Nurse Specialist, Hematology/Oncology/Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago
- Disclosure: Ms. Danner-Koptik has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion. Read Karina Danner-Koptik's profile.
Dr. Mary Nevin, Course Director; Vita Lerman, Planning Committee Member; Dr. John X. Thomas, Senior Associate Dean for Medical Education; Genevieve Napier, CME Director, Tara Scavelli and Jennifer Banys, CME Project Specialist have nothing to disclose.
Other Disclosure Information
At the conclusion of this activity, participants will be able to:
- Identify the most common long-term effects in adolescent and young adult cancer survivors
- Collaborate in the care of this population
- Identify and help remove barriers AYA cancer survivors face in transition to adult care
Estimated time to complete:
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- Read the article.
- Correctly answer at least 70% of questions on the quiz and answer evaluation questions.
Event-free survival rates for adolescents and young adults (AYAs) treated for cancer lag behind those of younger patients, even when treated on the same protocols. Following the completion of cancer treatment in a cancer center that served as their medical home, an effective transition to primary care is essential to achieve improved outcomes for AYAs. It is at this time that the general pediatrician or primary care provider is also crucial in partnering with patients, multidisciplinary providers and the cancer center to help care for AYAs and remove barriers to successful transition to the adult healthcare system. To help improve the care and outcomes of AYA cancer survivors, this article will review the most common long term effects of cancer treatments, discuss appropriate follow-up care for this population, and help primary care providers address challenges to effective transition to adult healthcare systems.
The National Comprehensive Cancer Network (NCCN) recently issued practice guidelines on the treatment of adolescents and young adults (AYAs) with cancer, including survivorship care and screening recommendations (Version 1.2012, released February 2012 http://www.nccn.org/professionals/physician_gls/pdf/aya.pdf). In the NCCN guidelines, AYAs are defined as patients 15-39 years of age at the initial cancer diagnosis. NCCN emphasizes that AYAs have unique needs and challenges that physicians need to recognize in order to improve outcomes.
The diagnosis of cancer in adolescence poses a unique developmental dilemma. On the one hand, the task of adolescence is to definitively separate and individuate from parents; on the other hand, a diagnosis of cancer places a teenager back in the dependent role of a sick child. Additionally, being diagnosed with cancer makes a teenager different from his/her peers at a time when fitting in is crucial to the development of self-esteem, positive body-image, and beginning skills to engage in intimate relationships. Lastly, adolescents have the cognitive ability to understand the implications of mortality of a cancer diagnosis, but may have no one with whom to discuss their concerns.
For reasons that are not fully clear, AYA cancer survivors have significantly worse outcomes and lower event-free survival rates than younger patients. Even when treated on the same pediatric protocols at pediatric institutions, event-free survival in AYAs is 64% compared to over 75% in younger children. Biological differences, potentially hormonal, are suspected. At this stage, research has mostly focused on the higher toxicity from cancer medications in AYA patients. Another factor that may contribute to worse outcomes in this age group is that over 50% of AYA cancer survivors do not participate in survivorship follow-up care, which needs to continue throughout adulthood.
Following the completion of cancer treatment, when the frequency of clinic visits lessen, AYAs may feel isolated and disconnected from their treatment team. Primary care providers then become ever more important in the care of AYA cancer survivors, making sure that they receive all the usual screenings and counseling about high risk behaviors recommended for all adolescents, as well as screening for various late effects of cancer therapies. These may include early onset of common adult health issues, such as osteoporosis and high cholesterol, as well as menopause, which sometimes can start soon after cancer treatment ends.
Long-term effects in AYA cancer survivors
AYA survivors of pediatric cancer have undergone intensive treatment with some combination of chemotherapy-radiation therapy-surgery that places them at risk for multiple late effects into adulthood. These include:
- Neurocognitive deficits and problems at school
- Bone density loss leading to early onset of osteoporosis
- Cardiac problems, such as cardiomyopathy and heart failure
- Pulmonary problems
- Endocrine abnormalities, such as decreased thyroid function
- Insulin resistance/metabolic syndrome, even in teens who are not overweight
- Musculoskeletal abnormalities
- Renal failure
- Secondary malignancies
- Psychosocial issues
Primary care providers need to be particularly aware of the potential impact of neurocognitive late effects as adolescent cancer survivors transition into adulthood. One study matched adolescents undergoing cancer treatment (excluding brain tumors) to their classmates and followed them over time. Survivors were more likely than their peers to report repeating a grade and had more school absences, but were similar to peers on developing an occupational self-concept and academic competence. Additionally, survivors and peers had similar success in graduating from high school, attaining employment, and expressing plans to pursue further education or seek employment opportunities. However, treatment intensity and severity of late effects were associated with multiple indices of educational and occupational attainment, so major vocational interventions may not be necessary for all cancer survivors, but further research is needed to identify subgroups at risk.
Adult survivors of childhood cancer also are twice as likely as healthy controls to be unemployed. A report from the Childhood Cancer Survivor Study showed that adult survivors of childhood cancer are employed in lower skilled jobs than siblings, who were used as a control group. This same study revealed that survivors with certain treatment history traits are at a higher risk for lower skilled jobs and may need vocational assistance throughout adulthood.
It is important to keep in mind that the neurocognitive late effects in AYA cancer survivors may emerge over time. These might include difficulties with memory, reading comprehension, information processing speed, and executive function deficits, such as the ability to prioritize and self-regulate, especially in less structured environments. Affected teens will need to learn how to manage these issues as they complete high school and prepare for college and work. Pediatricians should watch for signs of these deficits in order to intervene as early as possible. For example, an adolescent cancer survivor who suddenly starts acting out in school might be manifesting these late effects, and if identified early can get appropriate support services in school. Special education services and vocational help might be required.
The NCCN guidelines recommend that AYA survivors undergo continued screening in the following areas:
- Along the neuroendocrine axis
- Neuropsychological testing
- Females undergo breast cancer screening
- Cardiovascular risk assessment and screening
- Cardiomyopathy/asymptomatic heart failure screening
- Valvular heart disease screening
- Pulmonary screening
- Thyroid screening
- Colorectal cancer screening
- Kidney/bladder disease screening
- Gonadal function screening
- Screening for treatment related leukemias or myelodysplasias
- Audiology screening
Primary care providers also need to help AYA cancer survivors navigate the multitude of follow-up specialty services.
Collaboration with cancer center for follow-up care
Lifelong risk-based follow-up care that AYA cancer survivors require depends on individualized therapeutic exposures. Pediatricians are encouraged to contact the treating cancer center to request a “Treatment Plan/Summary” that will inform the specific follow-up needed for each patient. Risk-based long-term follow-up guidelines for survivors of childhood, adolescent and young adult cancers are available from the Children’s Oncology Group (Version 3.0, released October 2008 http://www.survivorshipguidelines.org/pdf/ltfuguidelines.pdf).
Many cancer centers offer survivorship programs that can partner with patients and primary care providers to ensure appropriate follow-up care. For example, the STAR (Survivors Taking Action & Responsibility) Program at Ann & Robert H. Lurie Children’s Hospital of Chicago is focused on comprehensive medical care for childhood cancer survivors, encompassing patient screening and education, ongoing research and health maintenance. A comprehensive clinical summary is developed when the patient is first seen in the STAR program via the Passport For Care™(PFC), which is a web-based clinical summary tool developed specifically for childhood cancer survivors by the Center for Collaborative and Interactive Technologies at Baylor College of Medicine and Texas Children’s Cancer Center with the Children’s Oncology Group (C.O.G.). The PFC folds in the appropriate screening guidelines and rationales for evaluation for individual patients based upon the current C.O.G. Survivorship Guidelines. An individual guideline is created for each patient based on his/her therapeutic exposures. This summary is then used by clinicians, not only as a guide for screening and evaluation, but also as an educational tool for the patient and family. This comprehensive summary is updated with each annual STAR visit and the family takes a copy with them. It is also embedded in the communication that goes back to the members of the patient’s healthcare team after each visit.
In addition to helping determine individualized follow-up plans, survivorship programs at cancer centers serve as coordination hubs for the multiple specialists involved in monitoring and treating late effects. Collaboration with a cancer survivorship program also can help an AYA survivor access early interventions and services when a primary care provider notices signs of cognitive or psychosocial late effect deficits.
Transitioning to adult care
It has been reported that disease prevention and management of AYA cancer survivors depends upon the transition to and adherence to the adult healthcare home. Therefore, it behooves us as clinicians to be vigilant in our education of the AYA survivors and steadfast in continuing the transition process, over time, as we care for them.
Transition to adult care is difficult for any young person with chronic health issues; for survivors of childhood cancer, that transition may be especially difficult. The American Academy of Pediatrics has supported the idea that AYAs may optimize their health and ability to manage their diseases with a well-planned transition.
Transition is a process, over time, which purposefully moves the AYA from the pediatric setting to appropriate adult healthcare. The broad goal of transition is for the AYAs to achieve successful engagement in the adult system, to receive developmentally and medically appropriate care, and to facilitate their maximum potential and optimal quality of life. The process should be coordinated, family centered and planned, so the AYA can mature into the knowledge of the transition to adult care and have supports in place to achieve the transition goal.
Patients will gradually need to assume more medical autonomy and this increased responsibility for their own healthcare could start in early adolescence. Developmental maturity must be taken into account during the transition process. For example, adolescents under 17 years of age are still impulsive in their decision-making process, whereas patients over 18 years of age may be better able to adhere to medical advice about their health.
Unfortunately, there are barriers which can alter or even halt the transition process for AYA cancer survivors. Transition from the pediatric oncology team to the adult healthcare team happens typically during a time of health insurance flux and instability. Many AYAs will lose their ability to remain on Medicaid plans, or may have sub-optimal health insurance coverage. Choosing appropriate health insurance coverage may be difficult and confusing for the AYA as our nation changes how coverage is accessed. The adult healthcare system is also focused on older adults, as they are the fastest growing population demographic. This can translate into decreased supports for the AYA, and also a perception that there is an abrupt termination of pediatric based services. This can leave the AYA and family feeling lost and often “falling through the cracks,” leading to insufficient follow-up care.
Close collaboration between the patient, primary care provider and a survivorship program or cancer center can help address many of these transition barriers. Partnering with a survivorship program can facilitate access to adult specialists and help with care coordination, making sure that important information about the survivor’s cancer treatment and follow-up plan is shared among the new adult providers.
For more information and patient education materials, see the online survivorship resources in Table 1 below.
Table 1: Web Resources for Childhood Cancer Survivors
American Childhood Cancer Organization
(formerly Candlelighters Childhood Cancer Foundation)
Provides information, awareness, advocacy and research for childhood cancer patients and families
Dedicated to providing transplant patients, survivors and their loved ones with emotional support and high quality, easy-to-understand information about bone marrow, peripheral blood stem cell and cord blood transplants
Provides free support services to anyone affected by cancer
|Children’s Oncology Group
Long-term follow-up guidelines for survivors of childhood, adolescent and young adult cancers
Unites the Children’s Oncology Group and the National Childhood Cancer Foundation to fund research
Nonprofit organization dedicated to providing reproductive information, support and hope to cancer patients and survivors whose medical treatments present the risk for infertility
|Lance Armstrong Foundation
Unites people to fight cancer and pursue an agenda focused on prevention, access to screening and care and improvement of the quality of life for cancer survivors
|Leukemia & Lymphoma Society
Health organization dedicated to funding blood cancer research, education
and patient services
|National Bone Marrow Transplant Link
Helps patients, caregivers and families cope with the social and emotional challenges of bone marrow/stem cell transplant from diagnosis through survivorship by providing vital information and personalized support services
|National Cancer Institute
Conducts and supports research, training, health information dissemination and other programs related to cancer diagnosis and treatment
|National Children’s Cancer Society
Provides support, information and education to those impacted by childhood cancer
|National Marrow Donor Program
A nonprofit organization dedicated to creating an opportunity for all patients to receive the bone marrow or umbilical cord blood transplant they need, when they need it
A national interdisciplinary initiative designed to explore the reproductive future of cancer survivors
An online community of young adults with cancer
Assists young adult survivors of cancer by providing financial support through the distribution of grants and scholarships
|STAR (Survivors Taking Action and Responsibility) Program
www.luriechildrens.org at Ann & Robert H. Lurie Children’s Hospital of Chicago
at Robert H. Lurie Comprehensive Cancer Center of Northwestern University